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Kluver-Bucy syndrome

MedGen UID:
124361
•Concept ID:
C0270707
•
Mental or Behavioral Dysfunction
Synonyms: Bilateral temporal lobe disorder; Memory loss, extreme sexual behavior, placidity, and visual distractibility; Post-encephalitic Kluver Bucy syndrome (type); Post-traumatic Kluver Bucy syndrome (type)
SNOMED CT: Klüver-Bucy syndrome (10651001)
 
Monarch Initiative: MONDO:0005817
Orphanet: ORPHA157823

Definition

A rare neurologic disease characterized by visual agnosia, hyperorality (strong tendency to examine objects orally), hypermetamorphosis (described as the irresistible impulse to notice and react to everything within sight), hypersexuality, changes in dietary habits and hyperphagia, placidity, and amnesia, due to bilateral lesions of the temporal lobe including the hippocampus and amygdala. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKluver-Bucy syndrome
Follow this link to review classifications for Kluver-Bucy syndrome in Orphanet.

Professional guidelines

PubMed

Klüver-Bucy Syndrome Following Traumatic Brain Injury: A Systematic Synthesis and Review of Pharmacological Treatment From Cases in Adolescents and Adults.
Clay FJ, Kuriakose A, Lesche D, Hicks AJ, Zaman H, Azizi E, Ponsford JL, Jayaram M, Hopwood M
J Neuropsychiatry Clin Neurosci 2019 Winter;31(1):6-16. Epub 2018 Oct 31 doi: 10.1176/appi.neuropsych.18050112. PMID: 30376788
Neuropsychiatric features of frontotemporal dementia: evaluation of consensus criteria and review.
Mendez MF, Perryman KM
J Neuropsychiatry Clin Neurosci 2002 Fall;14(4):424-9. doi: 10.1176/jnp.14.4.424. PMID: 12426410
Klüver-Bucy syndrome. Successful treatment with carbamazepine.
Hooshmand H, Sepdham T, Vries JK
JAMA 1974 Sep 23;229(13):1782. PMID: 4479148

Recent clinical studies

Etiology

Impaired acquisition of new words after left temporal lobectomy despite normal fast-mapping behavior.
Warren DE, Tranel D, Duff MC
Neuropsychologia 2016 Jan 8;80:165-175. Epub 2015 Nov 23 doi: 10.1016/j.neuropsychologia.2015.11.016. PMID: 26617264Free PMC Article
Comparison of therapeutic effects between selective amygdalohippocampectomy and anterior temporal lobectomy for the treatment of temporal lobe epilepsy: a meta-analysis.
Kuang Y, Yang T, Gu J, Kong B, Cheng L
Br J Neurosurg 2014 Jun;28(3):374-7. Epub 2013 Oct 7 doi: 10.3109/02688697.2013.841854. PMID: 24099101
The functional anatomy of non-verbal (pitch memory) function in left and right anterior temporal lobectomy patients.
Tracy JI, Hernandez RN, Mayekar S, Osipowicz K, Corbett B, Pascua M, Sperling MR, Sharan AD
Clin Neurol Neurosurg 2013 Jul;115(7):934-43. Epub 2012 Sep 30 doi: 10.1016/j.clineuro.2012.09.019. PMID: 23031747Free PMC Article
Transient News Events Test: feasibility in assessment of post-temporal lobectomy remote memory deficits.
Leeman BA, Macklin EA, Schomer DL, O'Connor MG
Epilepsy Behav 2009 Sep;16(1):113-9. Epub 2009 Jul 29 doi: 10.1016/j.yebeh.2009.06.012. PMID: 19643674
Clinical aspects of argyrophilic grain disease.
Ikeda K, Akiyama H, Arai T, Matsushita M, Tsuchiya K, Miyazaki H
Clin Neuropathol 2000 Nov-Dec;19(6):278-84. PMID: 11128620

Diagnosis

Mesiotemporal Disconnection and Hypoactivity in Klüver-Bucy Syndrome: Case Series and Literature Review.
Caro MA, Jimenez XF
J Clin Psychiatry 2016 Aug;77(8):e982-8. doi: 10.4088/JCP.14r09497. PMID: 27380585
A case of utilization behavior and hyperorality following bilateral anterior cerebral artery infarct partially responsive to carbamazepine: can both behaviors be attributed to lesions in different frontal lobe circuits?
Spiegel DR, Lamm K
Psychosomatics 2011 Nov-Dec;52(6):563-7. Epub 2011 Sep 28 doi: 10.1016/j.psym.2011.01.040. PMID: 22054627
Transient News Events Test: feasibility in assessment of post-temporal lobectomy remote memory deficits.
Leeman BA, Macklin EA, Schomer DL, O'Connor MG
Epilepsy Behav 2009 Sep;16(1):113-9. Epub 2009 Jul 29 doi: 10.1016/j.yebeh.2009.06.012. PMID: 19643674
Neuropsychiatric features of frontotemporal dementia: evaluation of consensus criteria and review.
Mendez MF, Perryman KM
J Neuropsychiatry Clin Neurosci 2002 Fall;14(4):424-9. doi: 10.1176/jnp.14.4.424. PMID: 12426410
Familial frontotemporal dementia associated with a novel presenilin-1 mutation.
Tang-Wai D, Lewis P, Boeve B, Hutton M, Golde T, Baker M, Hardy J, Michels V, Ivnik R, Jack C, Petersen R
Dement Geriatr Cogn Disord 2002;14(1):13-21. doi: 10.1159/000058328. PMID: 12053127

Therapy

Prevalence of hyperphagia in Alzheimer's disease: a meta-analysis.
Shea YF, Lee SC, Chu LW
Psychogeriatrics 2018 Jul;18(4):243-251. Epub 2018 Feb 6 doi: 10.1111/psyg.12316. PMID: 29409159
Comparison of therapeutic effects between selective amygdalohippocampectomy and anterior temporal lobectomy for the treatment of temporal lobe epilepsy: a meta-analysis.
Kuang Y, Yang T, Gu J, Kong B, Cheng L
Br J Neurosurg 2014 Jun;28(3):374-7. Epub 2013 Oct 7 doi: 10.3109/02688697.2013.841854. PMID: 24099101
The functional anatomy of non-verbal (pitch memory) function in left and right anterior temporal lobectomy patients.
Tracy JI, Hernandez RN, Mayekar S, Osipowicz K, Corbett B, Pascua M, Sperling MR, Sharan AD
Clin Neurol Neurosurg 2013 Jul;115(7):934-43. Epub 2012 Sep 30 doi: 10.1016/j.clineuro.2012.09.019. PMID: 23031747Free PMC Article
Superior colliculus lesions impair threat responsiveness in infant capuchin monkeys.
Maior RS, Hori E, Barros M, Teixeira DS, Tavares MC, Ono T, Nishijo H, Tomaz C
Neurosci Lett 2011 Oct 31;504(3):257-60. Epub 2011 Sep 28 doi: 10.1016/j.neulet.2011.09.042. PMID: 21970966
Mycoplasma pneumoniae central nervous system infections.
Daxboeck F
Curr Opin Neurol 2006 Aug;19(4):374-8. doi: 10.1097/01.wco.0000236617.04958.60. PMID: 16914976

Prognosis

Mesiotemporal Disconnection and Hypoactivity in Klüver-Bucy Syndrome: Case Series and Literature Review.
Caro MA, Jimenez XF
J Clin Psychiatry 2016 Aug;77(8):e982-8. doi: 10.4088/JCP.14r09497. PMID: 27380585
A case of utilization behavior and hyperorality following bilateral anterior cerebral artery infarct partially responsive to carbamazepine: can both behaviors be attributed to lesions in different frontal lobe circuits?
Spiegel DR, Lamm K
Psychosomatics 2011 Nov-Dec;52(6):563-7. Epub 2011 Sep 28 doi: 10.1016/j.psym.2011.01.040. PMID: 22054627
Superior colliculus lesions impair threat responsiveness in infant capuchin monkeys.
Maior RS, Hori E, Barros M, Teixeira DS, Tavares MC, Ono T, Nishijo H, Tomaz C
Neurosci Lett 2011 Oct 31;504(3):257-60. Epub 2011 Sep 28 doi: 10.1016/j.neulet.2011.09.042. PMID: 21970966
Neuropsychiatric features of frontotemporal dementia: evaluation of consensus criteria and review.
Mendez MF, Perryman KM
J Neuropsychiatry Clin Neurosci 2002 Fall;14(4):424-9. doi: 10.1176/jnp.14.4.424. PMID: 12426410
Clinical aspects of argyrophilic grain disease.
Ikeda K, Akiyama H, Arai T, Matsushita M, Tsuchiya K, Miyazaki H
Clin Neuropathol 2000 Nov-Dec;19(6):278-84. PMID: 11128620

Clinical prediction guides

Mesiotemporal Disconnection and Hypoactivity in Klüver-Bucy Syndrome: Case Series and Literature Review.
Caro MA, Jimenez XF
J Clin Psychiatry 2016 Aug;77(8):e982-8. doi: 10.4088/JCP.14r09497. PMID: 27380585
The neuropsychology of the Klüver-Bucy syndrome in children.
Lippe S, Gonin-Flambois C, Jambaqué I
Handb Clin Neurol 2013;112:1285-8. doi: 10.1016/B978-0-444-52910-7.00051-9. PMID: 23622339
Superior colliculus lesions impair threat responsiveness in infant capuchin monkeys.
Maior RS, Hori E, Barros M, Teixeira DS, Tavares MC, Ono T, Nishijo H, Tomaz C
Neurosci Lett 2011 Oct 31;504(3):257-60. Epub 2011 Sep 28 doi: 10.1016/j.neulet.2011.09.042. PMID: 21970966
Postinfectious immune-mediated encephalitis after pediatric herpes simplex encephalitis.
De Tiège X, De Laet C, Mazoin N, Christophe C, Mewasingh LD, Wetzburger C, Dan B
Brain Dev 2005 Jun;27(4):304-7. doi: 10.1016/j.braindev.2004.07.007. PMID: 15862196
Unsolved mysteries: the hippocampus.
Isaacson RL
Behav Cogn Neurosci Rev 2002 Jun;1(2):87-107. doi: 10.1177/1534582302001002001. PMID: 17715588

Recent systematic reviews

Klüver-Bucy Syndrome Following Traumatic Brain Injury: A Systematic Synthesis and Review of Pharmacological Treatment From Cases in Adolescents and Adults.
Clay FJ, Kuriakose A, Lesche D, Hicks AJ, Zaman H, Azizi E, Ponsford JL, Jayaram M, Hopwood M
J Neuropsychiatry Clin Neurosci 2019 Winter;31(1):6-16. Epub 2018 Oct 31 doi: 10.1176/appi.neuropsych.18050112. PMID: 30376788

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